Pulmonary lymphangiomyomatosis. Report of a case with immunohistochemical and ultrastructural findings.

Abstract

Pulmonary lymphangiomyomatosis is a rare disease characterized by wide-spread proliferation of smooth muscle cells around lymphatic and blood vessels, smaller airways and in the interstitium. Young women in the fertile status are almost exclusively affected. Spontaneous recurrent pneumothorax is the most important symptom. Chylous effusions are reported in 37.5% of cases. Our observation deals with a woman aged 43. Light- and electron-microscopic findings are described. Estrogen receptors have been detected on smooth muscle cells. Bilateral oophorectomy and treatment with progesterone were successful.